John W. Moore

Patent Ductus Arteriosus

The patent ductus arteriosus (PDA) is a vascular structure that connects the proximal descending aorta to the roof of the main pulmonary artery near the origin of the left branch pulmonary artery. This essential fetal structure normally closes spontaneously after birth. After the first few weeks of life, persistence of ductal patency is abnormal. The physiological impact and clinical significance of the PDA depend largely on its size and the underlying cardiovascular status of the patient. The PDA may be “silent” (not evident clinically but diagnosed incidentally by echocardiography done for a different reason), small, moderate, or large. Regardless of the size, complications may arise, and it is important for both pediatric and adult cardiologists to have an understanding of the pathophysiology, clinical implications, and management of PDA. The ductus arteriosus is a normal and essential fetal structure that becomes abnormal if it remains patent after the neonatal period. In normal cardiovascular development, the proximal portions of the sixth pair of embryonic aortic arches persist as the proximal branch pulmonary arteries, and the distal portion of the left sixth arch persists as the ductus arteriosus, connecting the left pulmonary artery with the left dorsal aorta (Figure 1). Normally, the distal right sixth aortic arch loses its connection to the dorsal aorta and degenerates. This transformation is complete by 8 weeks of fetal life. Figure 1. Schematic of embryonic aortic arch system. The 6 pairs of embryonic aortic arches are demonstrated (left-sided arches are numbered). The portions that normally involute are indicated by broken lines. The distal left sixth embryonic arch normally persists and becomes the PDA, connecting the left pulmonary artery to the proximal descending aorta. The right distal sixth arch normally involutes, as does the eighth segment of the right dorsal aorta (*), which results in a leftward aortic …

Percutaneous closure of the small patent ductus arteriosus using occluding spring coils

OBJECTIVES This report summarizes our experience with the use of occluding spring coils to close the small patent ductus arteriosus. BACKGROUND Several patent ductus arteriosus occluders (most notably the Rashkind device) have been developed and studied. Occluding spring coils have been used to close abnormal vessels and vascular connections. We previously reported the use of occluding spring coils to close the small patent ductus arteriosus in a small group of patients. This report describes our series of patients having patent ductus arteriosus closure with occluding spring coils. METHODS Between June 1990 and June 1993, 30 patients underwent cardiac catheterization to have patent ductus arteriosus closure by occluding spring coils. Selection criteria were age > 6 months and narrowest patent ductus arteriosus internal dimension < or = 3.0 mm by color flow imaging. Definitive selection was based on review of aortograms performed at catheterization. A 5.2F coronary catheter was used to deliver one or two standard occluding spring coils. A loop was delivered in the main pulmonary artery, and the remainder of the coil was delivered across the patent ductus arteriosus and into the aortic diverticulum. Patent ductus arteriosus closure was confirmed by aortography or color flow imaging, or both. Follow-up after coil placement occurred at 6 weeks and 6 months and included two-view chest radiography, echocardiography and color flow imaging. RESULTS Of the 30 patients, 29 had successful implantation by one (27 patients) or two (2 patients) occluding spring coils. Of these 29 patients, 19 had a clinically apparent and 10 had a silent patent ductus arteriosus. Average ductus minimal internal dimension was 1.7 mm (range 1.0 to 3.0). Complete closure of the ductus was confirmed in 27 patients by aortography or color flow imaging or both (in 24 within 4 h, in 2 after 6 weeks and in 1 after 6 months). Six weeks after implantation, two patients had a tiny residual patent ductus arteriosus noted on color flow imaging. One patient did not have successful implantation. This patient had a 3.2-mm ductus, and two coils migrated to the distal left pulmonary artery and could not be retrieved. There were no deaths or any significant complications noted during early or late follow-up in these patients. CONCLUSIONS Occluding spring coils may have additional application in closing the small patent ductus arteriosus.

Cardiovascular health supervision for individuals affected by duchenne or becker muscular dystrophy

Duchenne muscular dystrophy is the most common and severe form of the childhood muscular dystrophies. The disease is typically diagnosed between 3 and 7 years of age and follows a predictable clinical course marked by progressive skeletal muscle weakness with loss of ambulation by 12 years of age. Death occurs in early adulthood secondary to respiratory or cardiac failure. Becker muscular dystrophy is less common and has a milder clinical course but also results in respiratory and cardiac failure. The natural history of the cardiomyopathy in these diseases has not been well established. As a result, patients traditionally present for cardiac evaluation only after clinical symptoms become evident. The purpose of this policy statement is to provide recommendations for optimal cardiovascular evaluation to health care specialists caring for individuals in whom the diagnosis of Duchenne or Becker muscular dystrophy has been confirmed.

Adverse event rates in congenital cardiac catheterization — A multi-center experience†

Objectives: To describe case mix variation among institutions, and report adverse event rates in congenital cardiac catheterization by case type. Background: Reported adverse event rates for patients with congenital heart disease undergoing cardiac catheterization vary considerably, due to non‐comparable standards of data inclusion, and highly variable case mix. Methods: The Congenital Cardiac Catheterization Outcomes Project (C3PO) has been capturing case characteristics and adverse events (AE) for all cardiac catheterizations performed at six pediatric institutions. Validity and completeness of data were independently audited. Results: Between 2/1/07 and 4/30/08, 3855 cases (670 biopsy, 1037 diagnostic, and 2148 interventional) were recorded, median number of cases per site 480 (308 to 1526). General anesthesia was used in 70% of cases (28 to 99%), and 22% of cases (15 to 26%) were non‐electively or emergently performed. Three institutions performed a higher proportion of interventions during a case, 72 to 77% compared to 56 to 58%. The median rate of AE reported per institution was 16%, ranging from 5 to 18%. For interventional cases the median rate of AE reported per institution was 19% (7 to 25%) compared to 10% for diagnostic cases (6 to 16%). The incidence of AE was significantly higher for interventional compared to diagnostic cases (20% vs 10%, p<0.001), as was the incidence of higher severity AE (9% vs 5%, p<0.001). Adverse events in biopsy cases were uncommon. Conclusions: In this multi‐institutional cohort, the incidence of AE is higher among interventional compared to diagnostic cases, and is very low among biopsy cases. Equitable comparisons among institutions will require the development and application of risk adjustment methods.

Embolization and retrieval of the Amplatzer septal occluder

Embolization and percutaneous retrieval of the Amplatzer septal occluder (ASO) after release have been reported. However, the incidence, the causes of embolization, and the methods for effective retrieval have not been systematically described. In a survey of the ASO company‐designated proctors, the incidence of ASO embolization in this groups experience was 0.55% (21 embolizations in 3,824 device placements) with a wide range of patient demographics, atrial septal defect (ASD) sizes, and device sizes. Most embolizations occurred because of inadequate rim or undersized devices. Of the 21 embolizations, 15 of the devices were retrieved percutaneously with a gooseneck snare without morbidity or mortality. Six were retrieved at surgery. Of the 21 patients, 12 had ASO closure of their ASDs, and 9 had surgical ASD closure. In vitro, all devices could be retrieved with sheathes 2 Fr sizes larger than their recommended delivery sheath. Any device larger than 26 mm could be retrieved with its delivery sheath. The ability to pull the snared button into a sheath was variable and was assisted by pulling the device from above with a bioptome and by using a rigid notched sheath. Because the incidence of ASO embolization is about 1 in 200 in the most experienced hands, all operators should be prepared with the techniques and equipment required for percutaneous ASO retrieval. Catheter Cardiovasc Interv 2004;61:543–547.

2012 American College of Cardiology Foundation/Society for Cardiovascular Angiography and Interventions Expert Consensus Document on Cardiac Catheterization Laboratory Standards update: A report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents

published online May 8, 2012; J. Am. Coll. Cardiol. L. Tommaso Carl Moliterno, John W.M. Moore, Sandra M. Oliver-McNeil, Jeffrey J. Popma, and Landzberg, Warren K. Laskey, Charles R. McKay, Julie M. Miller, David J. Cavendish, Charles E. Chambers, James Bernard Hermiller, Jr, Scott Kinlay, Joel S. M. Bashore, MD, FACC,, Stephen Balter, Ana Barac, John G. Byrne, Jeffrey J. Documents, Society of Thoracic Surgeons, Society for Vascular Medicine, Thomas American College of Cardiology Foundation Task Force on Expert Consensus Catheterization Laboratory Standards Update Angiography and Interventions Expert Consensus Document on Cardiac 2012 American College of Cardiology Foundation/Society for Cardiovascular This information is current as of May 25, 2012 http://content.onlinejacc.org/cgi/content/full/j.jacc.2012.02.010v1 located on the World Wide Web at: The online version of this article, along with updated information and services, is

Catheterization for Congenital Heart Disease Adjustment for Risk Method (CHARM)

OBJECTIVES This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates. BACKGROUND The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007. METHODS A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios. RESULTS Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41. CONCLUSIONS Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease.

Fontan’s operation: is aspirin enough? Is coumadin too much?

BACKGROUND Thromboembolism after Fontans operation is attributed to low flow states, stasis in venous pathways, right to left shunts, blind cul-de-sacs, prosthetic materials, atrial arrhythmias, and hypercoagulable states. We assessed the efficacy of a strategy to reduce thromboembolic events including aspirin anticoagulation. METHODS From January 1996 through December 2000, 72 patients underwent Fontan procedures. Management included (1) avoidance of direct caval cannulation and central venous lines, (2) inotropic support for 48 to 72 hours to optimize cardiac output, (3) aortopulmonary anastomosis or suture closure of patent pulmonary valves, and (4) administration of aspirin (81 mg per day) beginning on postoperative day one. No other anticoagulation strategies were used. Surveillance included intraoperative and postoperative transesophageal echo, transthoracic echo at discharge, at first reevaluation, and at 6 month intervals, and catheterization 1 year after surgery. RESULTS There were no early or late deaths. Follow-up was completed with 2,882 patient-months and a mean of 40 months. There were no documented thromboembolic events; however, all suspicious occurrences were investigated by echo and brain imaging. There were no hemorrhagic events or aspirin-related complications. CONCLUSIONS Low dose aspirin can be used safely in young patients with Fontan connections. At intermediate follow-up, the strategies described appear effective in preventing thromboembolic complications. Routine use of more aggressive anticoagulation regimens seems unwarranted.

2012 American College of Cardiology Foundation/Society for Cardiovascular Angiography and Interventions expert consensus document on cardiac catheterization laboratory standards update: A report of the American College of Cardiology Foundation Task Force on Expert Consensus documents developed in collaboration with the Society of Thoracic Surgeons and Society for Vascular Medicine.

published online May 8, 2012; J. Am. Coll. Cardiol. L. Tommaso Carl Moliterno, John W.M. Moore, Sandra M. Oliver-McNeil, Jeffrey J. Popma, and Landzberg, Warren K. Laskey, Charles R. McKay, Julie M. Miller, David J. Cavendish, Charles E. Chambers, James Bernard Hermiller, Jr, Scott Kinlay, Joel S. M. Bashore, MD, FACC,, Stephen Balter, Ana Barac, John G. Byrne, Jeffrey J. Documents, Society of Thoracic Surgeons, Society for Vascular Medicine, Thomas American College of Cardiology Foundation Task Force on Expert Consensus Catheterization Laboratory Standards Update Angiography and Interventions Expert Consensus Document on Cardiac 2012 American College of Cardiology Foundation/Society for Cardiovascular This information is current as of May 25, 2012 http://content.onlinejacc.org/cgi/content/full/j.jacc.2012.02.010v1 located on the World Wide Web at: The online version of this article, along with updated information and services, is

Protein Aggregates and Novel Presenilin Gene Variants in Idiopathic Dilated Cardiomyopathy

Background— Heart failure is a debilitating condition resulting in severe disability and death. In a subset of cases, clustered as idiopathic dilated cardiomyopathy (iDCM), the origin of heart failure is unknown. In the brain of patients with dementia, proteinaceous aggregates and abnormal oligomeric assemblies of &bgr;-amyloid impair cell function and lead to cell death. Methods and Results— We have similarly characterized fibrillar and oligomeric assemblies in the hearts of iDCM patients, pointing to abnormal protein aggregation as a determinant of iDCM. We also showed that oligomers alter myocyte Ca2+ homeostasis. Additionally, we have identified 2 new sequence variants in the presenilin-1 (PSEN1) gene promoter leading to reduced gene and protein expression. We also show that presenilin-1 coimmunoprecipitates with SERCA2a. Conclusions— On the basis of these findings, we propose that 2 mechanisms may link protein aggregation and cardiac function: oligomer-induced changes on Ca2+ handling and a direct effect of PSEN1 sequence variants on excitation-contraction coupling protein function.